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Open access

[68Ga]-DOTATATE PET/CT and PET/MRI in the diagnosis and management of esthesioneuroblastoma: illustrative cases

Michelle Roytman, Andrew B. Tassler, Ashutosh Kacker, Theodore H. Schwartz, Georgiana A. Dobri, Sara B. Strauss, Alyssa M. Capalbo, Rajiv S. Magge, Marissa Barbaro, Eaton Lin, Joseph R. Osborne, and Jana Ivanidze

BACKGROUND

Esthesioneuroblastoma (ENB), also known as olfactory neuroblastoma, is a rare sinonasal neuroectodermal malignancy with a slow onset of symptoms, favorable 5-year survival, and a propensity for delayed locoregional recurrence. Current treatment options include resection, adjuvant radiotherapy, and/or chemotherapy; however, because of its rarity and location, determining the optimal treatment for ENB has been challenging.

OBSERVATIONS

ENBs strongly express somatostatin receptors (SSTRs), particularly SSTR2, providing a molecular target for imaging and therapy.

LESSONs

The authors present a case series of ENBs imaged with [68Ga]-DOTATATE PET/MRI and PET/CT and discuss the emerging role of [68Ga]-DOTATATE PET for ENB diagnosis, staging, and treatment response monitoring.

Open access

Papillary craniopharyngioma in a patient following resection of nonfunctioning pituitary adenoma: illustrative case

David J. Park, Akash Mishra, Danielle Golub, Jian Y. Li, Karen S. Black, and Michael Schulder

BACKGROUND

Although craniopharyngioma and pituitary adenoma are common tumors of the sellar or suprasellar region, the development of papillary craniopharyngioma in the same sellar region after resection of a nonfunctioning pituitary adenoma has not been reported.

OBSERVATIONS

Here the authors report the first case of craniopharyngioma that developed long after resection of a pituitary adenoma. A 66-year-old male patient underwent endoscopic transsphenoidal resection for a large sellar mass, which histopathologically confirmed the diagnosis of a pituitary adenoma. He had an excellent recovery after surgery. For several years, he had no clinical or imaging evidence of tumor recurrence and then was lost to follow-up. Seven years after the initial surgery, the patient returned with a one-month history of visual field defects, and imaging confirmed a heterogeneous, cystic suprasellar mass. Endoscopic transsphenoidal resection of the tumor was performed, and histological examination showed it to be a papillary craniopharyngioma.

LESSONS

Neurosurgeons should be aware that after pituitary adenoma resection, a recurrent mass could be a craniopharyngioma, with implications for very different management recommendations.

Open access

The recurrent plexiform neurofibroma of the scalp in neurofibromatosis type 1: illustrative case

Galih Indra Permana, M. Arifin Parenrengi, Wihasto Suryaningtyas, Dyah Fauziah, and Muhammad Azzam

BACKGROUND

Plexiform neurofibroma is a benign tumor of the peripheral nerves. It is an unusual variant of neurofibroma originating from all parts of the nerve. Plexiform neurofibroma is primarily pathognomonic and exhibits an unusual variant from neurofibromatosis type 1 (NF1). The possibility of malignancy and recurrence are the main reasons for long-term, close follow-up.

OBSERVATIONS

The authors report a case of a 14-year-old girl with a recurrent plexiform neurofibroma derived from the peripheral nerves, which also presented with a typical sign of NF1 disease. The aim of the tumor resection is symptomatic relief.

LESSONS

Accomplishing a good outcome can be related to good perioperative planning and a precise operative procedure. The result of anatomical pathology determines the prognosis of the patient. Clinical examination and radiological studies are needed to evaluate the recurrence of complications after surgical procedures.

Open access

A case of brain metastasis with pathological transformation of long-surviving malignant pleural mesothelioma: illustrative case

Ryuichi Noda, Shunsuke Yanagisawa, Masato Inoue, and Tetsuo Hara

BACKGROUND

Malignant pleural mesothelioma (MPM) is a rare cancer, and in 80% of cases the cause is asbestos exposure. In 1972, the World Health Organization (WHO) declared asbestos is a carcinogenic substance. Since then, every developed country has restricted and banned the product. Because of its high heat resistance, asbestos had been widely used as building material for decades. The WHO estimated that approximately 125 million people are exposed to asbestos, and more than 107,000 die from asbestos-related diseases annually. Because of its long incubation period, the number of patients is estimated to keep increasing in the near future.

OBSERVATIONS

The authors report a case of long-surviving MPM with a rushed clinical course after brain metastasis. A 69-year-old woman diagnosed with MPM (epithelial type) 6 years earlier presented with a brain metastasis. The pathological result of the brain metastasis was the sarcomatoid type. This case showed the possibility of subtype transition after long survival.

LESSONS

This article aids in understanding the long-term natural history of MPM and the possibility of epithelial-mesenchymal transition. Neurosurgeons have to be aware of its the natural history and the possibility of brain metastasis.

Open access

Intracranial temporal bone angiomatoid fibrous histiocytoma: illustrative case

Shivani Gillon, Jacqueline C. Junn, Emily A. Sloan, Nalin Gupta, Alyssa Reddy, and Yi Li

BACKGROUND

Angiomatoid fibrous histiocytoma (AFH) is a rare, slowly progressive neoplasm that most commonly occurs in soft tissues. AFH rarely occurs in bone such as the calvaria. The authors present a case of AFH in the petrous temporal bone, which, to their knowledge, is the first case of AFH in this location.

OBSERVATIONS

A 17-year-old girl presented with worsening positional headaches with associated tinnitus and hearing loss. Imaging demonstrated an extraaxial mass extending into the right cerebellopontine angle, with erosion of the petrous temporal bone, with features atypical for a benign process. The patient underwent retrosigmoid craniotomy for tumor resection. Pathology was consistent with a spindle cell tumor, and genetic testing further revealed an EWSR1 gene rearrangement, confirming the diagnosis of AFH. The patient was discharged with no complications. Her symptoms have resolved, and surveillance imaging has shown no evidence of recurrence.

LESSONS

The authors report the first case of AFH in the petrous temporal bone and only the second known case in the calvaria. This case illustrates the importance of the resection of masses with clinical and imaging features atypical of more benign entities such as meningiomas. It is important to keep AFH in the differential diagnosis for atypical masses in the calvaria and skull base.

Open access

Simultaneous intracranial and testicular germ cell tumors: illustrative case

Lei Han, Jie Lu, Luxiong Fang, Songtao Qi, and Ye Song

BACKGROUND

Simultaneous intracranial and testicular germ cell tumors (GCTs) are extremely rare, leading to a lack of adequate experience in their treatment. Therefore, the authors report a case of this kind of GCT.

OBSERVATIONS

A 5-year-old boy was admitted to the hospital with headache and vomiting. Computed tomography and magnetic resonance imaging suggested the possibility of a GCT in the pineal region. The value of the serum tumor marker alpha-fetoprotein (AFP) was 5,396.1 μg/L, and β-human chorionic gonadotropin levels were within the normal range. Subsequently, the tumor was removed, and the final pathological result was a mixed GCT. Therefore, chemotherapy and radiation were added. However, the authors found a testicular tumor on ultrasound at the same time, and pathology after surgery suggested a mature cystic teratoma. Following treatment, the patient recovered well, and AFP levels dropped to normal values.

LESSONS

To the authors’ knowledge, this report is the fourth case of simultaneous intracranial and testicular GCTs and the first case of a simultaneous mixed GCT in the pineal region and mature teratoma of the testis. A combination of surgery, chemotherapy, and radiation therapy for mixed GCTs in the pineal region and surgical excision for testicular reproductive cell tumors are effective in these patients, but long-term monitoring is required.

Open access

Spinal intramedullary hemangioblastoma and schwannoma collision tumor: illustrative case

Matthew T. Neal, Alexandra E. Richards, Kara L. Curley, Kliment Donev, Mark K. Lyons, and Maziyar A. Kalani

BACKGROUND

Intramedullary spinal cord tumors represent a minority of intradural tumors. Among intramedullary spinal cord tumors, hemangioblastomas are uncommon, and schwannomas are extremely rare. Collision tumors are histologically distinct tumors that are intermingled and growing together.

OBSERVATIONS

In this report, the authors describe a patient with a cervical intramedullary collision tumor involving a hemangioblastoma and schwannoma. To the authors’ knowledge, no prior spinal intramedullary collision tumor involving multiple neoplasms has been described. The patient’s presentation and management are described.

LESSONS

Clinicians should consider the possibility of collision tumors when evaluating intramedullary spinal cord tumors, especially when patient presentation and radiographic findings are atypical. When tumors with similar radiographic characteristics form collision tumors, distinction using preoperative imaging can be extremely challenging. In addition, surgical management of intramedullary collision tumors, like that for all intramedullary spinal cord tumors, should involve meticulous perioperative care and a methodical surgical technique. Maximal safe resection will depend upon histopathological diagnosis, anatomical location of the tumor, presence of distinct dissection planes, and stability of neuromonitoring. Finally, ongoing research on the genetics of intramedullary spinal cord tumors may identify underlying genetic links for intramedullary hemangioblastomas and schwannomas.

Open access

Misdiagnosis of elevation of β-hCG in cystic craniopharyngioma: illustrative case

Umaira Saleh, Liang Hooi Lim, Ihfaz Ismail, and Nasser Abd Wahab

BACKGROUND

Craniopharyngiomas and germ cell tumors (GCTs) are both rare intracranial tumors commonly present in childhood or middle age. They share similar clinical and radiological features. GCTs commonly give rise to tumor markers in the cerebrospinal fluid, hence guiding the treatment plan.

OBSERVATIONS

This article reports the case of a 5-year-old boy with a large sellar and suprasellar mass with obstructive hydrocephalus. Laboratory studies showed increased beta-human chorionic gonadotrophin (β-hCG) levels in the cystic fluid, suggestive of choriocarcinoma. He underwent 3 cycles of chemotherapy but showed a poor response. Further aspiration followed by tumor debulking was performed, and histopathological examination revealed craniopharyngioma.

LESSONS

This case report indicates that β-hCG, commonly regarded as a specific tumor marker for choriocarcinoma, is detectable in other forms of suprasellar tumors. The authors highlight clinical and radiological features of suprasellar tumors that can be misdiagnosed as intracranial GCTs. The relevance of tumor markers and indications for histopathological confirmation are discussed.

Open access

Cerebellar high-grade glioma with H3 K27M mutation: illustrative case

Mikiko Takahashi, Yusuke Tomita, Shoko Merrit Yamada, Kazuto Yamazaki, Hideaki Yokoo, Tatsuya Aso, and Masashi Kawamoto

BACKGROUND

Diffuse midline glioma H3 K27M-mutant is a distinct subtype of glial tumors newly introduced in the revised fourth edition of the World Health Organization Classification of Tumours of the Central Nervous System. They are aggressive pediatric tumors with a poor prognosis but have also been reported in adults.

OBSERVATIONS

The authors present the case of a man in his 60s who presented with rotatory vertigo, taste disorder, and right facial paralysis. Magnetic resonance imaging showed a tumor expanding from the cerebellum to the pons, which was histologically identified as glioblastoma, grade IV, IDH wild type. After tumor resection, the patient received chemoradiotherapy but showed only a partial response. His condition gradually worsened, and he died of progressive disease 12 months postoperation, after which an autopsy was performed. Tumor cells with a high nuclear-to-cytoplasm ratio were immunohistochemically analyzed and found to test positive for H3 K27M and negative for H3 K27me3. Furthermore, mutational analysis revealed HIST1H3B K27M mutation, and the tumor was finally identified as a high-grade glioma H3 K27M-mutant. The tumor invaded widely along the cerebral ventricle and disseminated to the spinal cord.

LESSONS

When a glioblastoma shows localization or dissemination patterns different from those of typical glioblastoma, an H3 K27M-mutant glioma should be suspected.

Open access

Falcine chondroma: illustrative case

Melissa M. J. Chua, Stanley F. Bazarek, David M. Meredith, Liangge Hsu, and Stephen C. Saris

BACKGROUND

Chondromas are benign cartilaginous tumors that are rarely seen in the brain.

OBSERVATIONS

A 58-year-old woman had undergone routine brain imaging after a motor vehicle accident and was incidentally found to have a right falcine lesion. Contrast magnetic resonance imaging showed a mostly nonenhancing mass with discontinuous rim enhancement. She was taken to the operating room and pathology revealed a chondroma.

LESSONS

Falcine intracranial chondromas are rare and typically misdiagnosed as meningiomas. Chondromas should be in the differential for patients presenting with nonenhancing falcine lesions.